Abstract
Peripheral nerve sheath tumors (PNSTs) are defined as type of sarcomas that develops in cells which forms a protective sheath (covering) around the peripheral nerve, i.e., the cells of myelin sheath. Nerve tumors are of neuroectodermal in origin as it was composed of small rounded ectodermal cells that affect exclusively soft tissues. PNSTs are most common neoplasm with classic clinicopathological features, but they are diagnostically challenging. They consist of wide spectrum of tumors ranging from benign tumors to malignant nerve sheath tumors and its prevalent in oral tissues. Diagnosis of PNSTs are quite hectic but made possible by histopathology and immunohistological markers.